[PAGID] <no subject>

[Christine Seroogy, M.D.]

My first thought is:  what is the HIV status in the mother?

Chris


Christine M. Seroogy MD, FAAP, FAAAAI
Associate Professor
University of Wisconsin
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
1111 Highland Avenue
4139 WIMR
Madison, WI  53705-2275
phone: 608-263-2652
fax: 608-265-9721




On 6/10/10 9:11 AM, "Routes, John" <jroutes at mcw.edu> wrote:


> I am posting this from Dr. AL-Zahrani for everyones input

> 

> 

> 

> 

> 

> Dear Dr. John Routes,

> 

> 

> I would appreciate it very much if you can help me with TREC analysis for

> difficult case that I have.

>    The case is a most 2 -month old transferred to our ICU form private

> hospital because of recurrent sepsis, candidemia, thrompocytopenia and

> elevated liver enzymes. She was treated aggressively with IV antibiotics and

> ampho-B.

> Her thrompcytpoenia resolved and liver enzymes are declining. She has no

> skin rash.

> They consulted our service because she developed K. pneumonia sepsis after

> resolution of her previous sepsis.

> Her immunologic work up showed (normal CD3+ NK+), but persistent B-cell

> lymphopenia ( 0.08 cells) while she continued to maintain normal level of

> her Ig's over the last 2-3 weeks(IgG=3.4 g/l , IgM= 0.7 g/l and IgA=0.5g/L)

> despite the serious  recurrent infections and No IVIG given.

> The PHA was normal (as the control); done 3 times ( once/week).

> CD3+CD4+CD45 RA+ =12%  and  CD3+CD4+CD45 RO+ = 40%.

> TCR  a/b  = 96% and TCR g/d = 4% ( of total T ­cells). Normal adhesion

> molecules.

> The CXR; showed absent thymus shadow.

> The 1st HIV-PCR was positive ( she received several PRCB and platelets

> transfusion in the referring Hospital), however, it was done 2 times

> thereafter and was negative.

> The ID colleagues recommend to repeat it 4-weeks late.

> EBV, CMV, HSV by PCR all negative.

> Some thing made the story more confusing is that, she was given

> Phenobarbital for seizure when she was in the private hospital.

> 

> Now could this be;

> 1)      A Leakly SCID (serous infections and absent thymus)?

> 2)      Or could hypomorous RAG1/RAG2 or artimus mutations present like this

> ?

> 3)      Or Phenobarbital related B-cell lymphopenia, as she is maintaining

> her Ig's and no infections for 2-wk Š?

> 4)      Or could HIV present like this at the beginning before she shows

> persistent viremia (high HIV-PCR).

> 

> Could you please help us by doing TREC analysis there after if it confirm

> SCID we can proceed with BMT.

> 

> I appreciate your response and help.

> 

> Thank you and have a nice day.

> 

> Sincerely,

> 

> DR. Daifulah AL-Zahrani, MD, FAAP, ABAI.

> Consultant allergy, Immunology and BMT.

> Pediatric Department.

> King Abdulaziz medical City ­WR

> P.O.Box: 9515

> Jeddah 21423

> Saudi Arabia

> Tel: +966-26240000 Ext: 22069

> Mobile: +966-505203231

> 

> 

>